Seminars in Thoracic and Cardiovascular Surgery ( IF 2.5 ) Pub Date : 2021-10-21 , DOI: 10.1053/j.semtcvs.2021.10.006 C Charles Jain 1 , Alexander C Egbe 1 , Elizabeth H Stephens 2 , Heidi M Connolly 1 , Donald J Hagler 3 , Moira B Hilscher 4 , William R Miranda 1
Patients with pulmonary atresia and intact ventricular septum (PA-IVS) require intervention early in life, and most survive to a definitive procedure of either Fontan circulation or right ventricle to pulmonary artery (RV-PA) repair. It remains unknown how surgical strategy impacts hemodynamics and comorbidities in adults. Retrospective analysis of adults (age ≥18 years) with PA-IVS undergoing hemodynamic catheterization at Mayo Clinic, MN between January 2000 through January 2020 was performed. 14 patients in the RV-PA group (71% biventricular, 29% 1.5 ventricle repair) and 19 post-Fontan patients [9 lateral tunnel (48%), 6 atriopulmonary (32%), and 4 extracardiac (21%)] were identified. Median age was 29 (21, 34) years. There were no differences in demographics and laboratory data (including MELD-XI) between groups. All patients assessed for liver disease had evidence of hepatic congestion or cirrhosis (14 in the Fontan group and 4 in the RV-PA group). Invasive hemodynamics were comparable between groups with the Fontan and RV-PA groups having similar systemic venous pressure (15.7±4.4 vs. 14.3±6.2, p = .44) and cardiac output (2.2±0.6 vs. 2.0±0.4 L/min/m2, p = .23). There was no difference in transplant-free survival (p = .92; 5-year transplant-free survival RV-PA 84%, Fontan 80%). Hemodynamic derangements, namely elevated systemic venous pressure and low cardiac output, are prevalent in patients with PA-IVS undergoing cardiac catheterization regardless of surgical strategy.
中文翻译:
无论采用何种修复策略,成人肺动脉闭锁和室间隔完整的导管插入术中普遍存在系统性静脉高压和低输出量
患有肺动脉闭锁和完整室间隔 (PA-IVS) 的患者需要在生命早期进行干预,并且大多数患者存活到 Fontan 循环或右心室的最终手术肺动脉 (RV-PA) 修复。目前尚不清楚手术策略如何影响成人的血流动力学和合并症。对 2000 年 1 月至 2020 年 1 月期间在明尼苏达州梅奥诊所接受血液动力学导管插入术的 PA-IVS 成人(年龄≥18 岁)进行了回顾性分析。RV-PA 组的 14 名患者(71% 双心室,29% 1.5 心室修复)和 19 名 Fontan 术后患者 [9 名侧隧道 (48%)、6 名房肺 (32%) 和 4 名心外 (21%)]确定。中位年龄为 29(21、34)岁。组间的人口统计学和实验室数据(包括 MELD-XI)没有差异。所有接受肝病评估的患者都有肝充血或肝硬化的证据(Fontan 组 14 名,RV-PA 组 4 名)。静脉压(15.7±4.4 对比 14.3±6.2,p = .44)和心输出量(2.2±0.6 对比 2.0±0.4 L/min/m 2 ,p = .23)。无移植存活率没有差异(p = .92;5 年无移植存活率 RV-PA 84%,Fontan 80%)。血流动力学紊乱,即全身静脉压升高和心输出量低,在接受心导管术的 PA-IVS 患者中很普遍,无论手术策略如何。